P1444: AVASCULAR NECROSIS IN PATIENTS WITH SICKLE CELL DISEASE: A RETROSPECTIVE ANALYSIS AT A SPECIALIST HAEMOGLOBINOPATHY CENTRE
Authors: Niamh Loughran, Jane Potter, Naeem Desai, Joe Sharif
Journal: HemaSphere (2023)

Topic: 26. Sickle cell disease Background: Avascular necrosis (AVN) is a well described complication of sickle cell disease (SCD). It most commonly affects the head of femur, other bones such as the head of the humerus can be affected. 2016 Cochrane database reports that up 50% of sickle patients have AVN. Prevention, recognition, and treatment is key to minimizing impairment and disability, and thus improving quality of life and reducing hospital admissions. There is a lack of treatment options for prevention of this complication and no clear consensus on the optimum timing of surgical intervention. There may also be a lack of specialists experienced in joint replacement in this cohort, which represent a younger population of joint replacements compared to the national average and with varying bone architecture. We examined the prevalence of AVN in adults with sickle cell disease at a single specialist haemoglobinopathy centre. As part of a patient’s routine review appropriate imaging is requested if symptoms are suggestive of AVN. Conservative management options are largely supportive including analgesia and physiotherapy. Joint replacement surgery requires careful consideration and multidisciplinary involvement. Aims: •Investigate the prevalence and demographic spread of AVN in the current adult sickle cell population •Identify what imaging and management options were employed, and any associated complications Methods: A retrospective analysis of all patients registered with sickle cell disease at our institution. Electronic patient records were used to identify patients with a documented diagnosis of AVN, affected site, age at diagnosis, imaging, documented grade of AVN, surgical management and any associated complications. Demographics including sickle cell genotype and gender were also collected, along with current sickle cell treatments. Results: 59/447(13%) sickle cell patients had a documented AVN diagnosis, 56% female: 44% male. 42 (71%) had femoral head involvement alone, 6 (10%) had humeral head involvement alone, and 11 (19%) had both. There was even distribution across HbSS and HbSC. 46 (78%) patients’ diagnosis was confirmed with MRI, none of which had a documented classification. 5 were diagnosed via x-ray and 8 had a diagnosis on registering as a patient in Manchester. A total of 24/59 (40%) patients underwent surgical intervention, with the primary intervention at a median age of 33 years (IQR 23-39) significantly younger than non-sickle cell patient national average of 69 years (IQR 61-76). 8/59 underwent decompression surgery, 3 of which went on to have THR. In total 16/59 had a THR and 3/59 had a shoulder replacement. Of the 16, 6/16 had no reported complications in the short or long term, 3/16 to date have required revision surgery, 6/16 have documented chronic pain 10/16 have other documented complications. 26/59 patients with reported AVN are currently on hydroxycarbamide, and 6/59 on regular transfusion programmes. Summary/Conclusion: We describe the prevalence of AVN in our sickle cell population which appears lower than other centres and a small proportion have required surgical intervention. Consensus on formal grading of severity of AVN may aid future management decisions. There is a lack of data on the natural history of AVN in SCD and a lack of treatment options for early disease. Early surgical intervention may result in improved quality of life but the lifespan of a prosthesis in a young patient along with risks of surgery must be taken into consideration. Keywords: Osteonecrosis, Sickle cell patient, Sickle cell disease